The complications of non-tropical sprue were registered in 100 patients seen during an 18-year period. The patients had a significantly higher mortality than the age- and sex-matched general population. They had an increased incidence of malignancies, predominantly malignant lymphomas and carcinomas of the gastrointestinal tract. The disease must be considered a premalignant condition.

The skin and oral mucosa were studied in an unselected series of carriers of x-linked chronic granulomatous disease, a hereditary condition in which phagocytic cells display a pronounced functional defect. Three carriers had discoid lupus erythematosus (DLE)-like skin lesions which histopathologically were consistent with DLE of the hypertrophic and profundus type. Four patients had experienced photosensitivity in childhood. Seven patients had recurrent aphthous-like stomatitis which should be distinguished from the recurrent aphthous stomatitis seen in otherwise healthy individuals. The remarkably high incidence of DLE-like symptoms in heterozygous carriers might be related to the presence of mixed populations of defective and normal phagocytes. The variable expression of skin symptoms may be related to uneven distribution of abnormal and normal phagocytes. Female patients with these clinical symptoms, especially the combination of DLE-like skin lesions and aphthous-like stomatitis, should be suspected of being carriers of chronic granulomatous disease and studies of phagocyte function in vitro should be performed, since the diagnosis of the carrier state is of utmost importance for genetic counselling before pregnancy.

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